Hypertrophic Pachymeningitis in Chinese Patients: Presentation, Radiological Findings, and Clinical Course

Abstract

Background: Hypertrophic pachymeningitis (HP) is generally regarded as a rare inflammatory disease, which results in a diffuse thickening of the dura mater. We retrospectively collected data from patients with HP.

Methods: A total of 16 patients with HP were included in our study. The clinical features, laboratory evaluation, imaging findings, treatment, and outcome were reviewed.

Results: Of the 16 cases, half were male, with a mean age of 52.6 ± 13.2 years. The mean duration from onset to diagnosis was 8.6 months. The most frequent presenting symptoms in HP cases were a recurrently chronic headache (81.3%) and multiple cranial nerve injury (50%). Antineutrophil cytoplasmic antibody- (ANCA-) related HP was found in 5 cases and IgG4-related HP in 1 case. The intracranial pressure was elevated in 4 cases. The cerebrospinal fluid (CSF) had lymphocytosis in 5 cases and increased protein in 12 cases. Immunoglobulins (IgG, IgA, and IgM) and protein showed linear relationships in the CSF. On magnetic resonance imaging (MRI), localized or diffuse dura maters were thickened in all cases. HP combined with subacute subdural hemorrhage or hypertrophic spinal pachymeningitis was also observed in individual cases. Biopsy of the dura mater in one case showed amounts of inflammatory cells infiltrating, with an increased percentage of IgG4-positive plasma cells. Of all cases referring to glucocorticoid treatment, the symptoms have improved significantly in 10 cases. In other 6 cases, mycophenolate mofetil or azathioprine was added. All patients showed clinical improvement at the follow-up visits.

Conclusion: The clinical characters of HP are chronic onset, recurrently chronic headache, and multiple cranial nerves paralysis. Inflammatory changes in CSF caused by intrathecal synthesis of immunoglobulin, characteristic dural enhancement on MRI, and pathologic biopsy are all helpful for diagnosis. The addition of immunosuppressant, especially mycophenolate mofetil, is a good choice for steroid-resistance HP.

Figure 1

Scatter plot show a linear correlation between immunoglobulin (IgG, IgA, and IgM) and protein in CSF.

Figure 2

Neuroimaging of hypertrophic pachymeningitis. Case 4. Axial and coronal T1-contrasted MRI reveal enhancement of thickened falx cerebrum and tentorium cerebellum, mimicking the logo of Mercedes Benz (“Benz” sign) in coronal MRI (a). Case 5. Sagittal and coronal T1-contrasted MRI show thickening of left tentorium cerebellum, left frontal, and temporal dura mater (b). Case 6. Axial and coronal T1-contrasted MRI show diffuse linear dural enhancement, including the falx cerebrum (c).

Figure 3

IgG4-related hypertrophic pachymeningitis involving the spinal dura mater (Case 8). Diffuse linear dural enhancement appears on axial (a) and coronal (b) T1-contrasted MRI. Sagittal (c) and axial (d) T1-contrasted MRI show dural thickening of the upper thoracic spinal dura mater. Pathologic slide of biopsy shows dense fibrosis and inflammatory cell infiltration (H&E 100×, (e)), with an increased percentage of IgG4-positive plasma cells (IgG4-IHC 100×, (f)). The dural enhancement is markedly reduced after steroid and immunosuppressive therapy (mycophenolate mofetil) on 4 mouths follow-up (g).

Figure 4

Hypertrophic pachymeningitis with subdural hemorrhage (Case 9). Bilateral subdural hemorrhage appears on axial T2-weighted fluid-attenuated inversion recovery imaging (a). Coronal T1-contrasted MRI reveals diffusion enhancement of the dura and right mastoiditis (b) and axial T1-contrasted MRI shows enhancement of the peripheral margin of the dura without central enhancement in frontal dura mater (c). Right otomastoiditis appears on MRI (b) and magnetic resonance venography was unremarkable (d).